Diseases & Conditions

• Bronchiectasis
• Cystic Fibrosis
• Chronic Obstructive Pulmonary Disease
• Cerebral Palsy
• ICU/Post Op Setting
• Muscular Dystrophy
• Primary Ciliary Dyskinesia
• Post-Lung Transplant
• Spinal Injury/Quadriplegia
• Spinal Muscular Atrophy
• Fact Sheets & Referral Listings

• What's new!
• Events Calendar
• About Us
• Fact Sheet
• Contact Us
• Home

Central Hypoventilation Syndrome

Central hypoventilation syndrome (also called Ondine's Curse) is a rare, severe form of central sleep apnea. In central hypoventilation syndrome, impairment of the central nervous system causes affected individuals to lack an adequate arousal response to the presence of increased carbon dioxide or to the lack of oxygen in the blood. In severely affected individuals, hypoventilation occurs both while awake and asleep. Not only is the automatic ventilatory response impaired in this patient population; the perception of asphyxia, or the behavioral awareness of hypoventilation, is absent as well. For this reason, life-long mechanical ventilatory support with concomitant placement of an artificial airway is usually required.

Placement of an artificial airway for the purpose of mechanical ventilation impairs airway clearance and increases the risk of pulmonary infection in a number of ways.

• Local irritation associated with intubation or tracheostomy results in excessive production of mucus. Mucus hypersecretion overburdens the mucociliary apparatus, resulting in congestion and plugging of small and large airways with static mucus.
• Presence of an artificial airway typically impairs cough function and may destroy cilia, further inhibiting normal airway clearance.
• The introduction of an artificial airway may lead to temporary glottic dysfunction and increased risk of aspiration. Aspirated bacteria from saliva and gastric contents enter normally sterile airways resulting in serious, potentially fatal pneumonia.
• Almost 90% of those who are intubated for long-term ventilation suffer from tracheal colonization. Such patients require frequent suctioning to maintain patency of the airway. Despite precautions, the suctioning may serve as the vector of deep infection, inoculating the lungs with tracheal bacteria.

Dependence on mechanical ventilation limits the use of exercise to maintain aerobic capacity, bellows function, and lung volume, thus increasing the risk of developing restrictive lung disease. The consequent inability to generate a large inspiratory or expiratory force or to cough effectively compromises mucociliary clearance and exposes patients to recurrent respiratory infection.

Ineffective secretion clearance associated with mechanical ventilator dependency results in impaired gas exchange and significant risk of bacterial colonization within the respiratory tract. Recurrent infection and progressive pulmonary damage can result in respiratory failure. Aggressive bronchial hygiene, including daily Airway Clearance Therapy, is an essential component in preventing or managing the pulmonary complications that contribute most significantly to morbidity and mortality among patients dependent upon mechanical ventilation.

Back to Referral Listing

Sources

Weese-Mayer D, Shannon D, Keens T, Silvestri J. Idiopathic congenital central hypoventilation syndrome. Am J Respir Crit Care Med. 1999; 160: 368-373.

Tung A. Indications for mechanical ventilation. Anesthesia Clin . 1997: 35(1): 1-17. Meduri GU, Estes RJ. The pathogenesis of ventilator-associated pneumonia. Intensive Care Med. 1995; 21: 365-383; 452-461.

Torres A, Anzar R, Gatell JM, Jimenez P, Gonzales J, et al. Incidence, risk, and prognosis factors of nosocomial pneumonia in mechanically ventilated patients. Am Rev Respir Dis. 1990; 142: 523-528.

500708 AA 10/00